title: Pathophysiologic and anesthetic considerations for patients with myotonia congenita or periodic paralyses.
abstract: Myotonia congenita and periodic paralyses are hereditary skeletal muscle channelopathies. In these disorders, various channel defects in the sarcolemma lead to a severely disturbed membrane excitability of the affected skeletal muscles. The clinical picture can range from severe myotonic reactions (e.g., masseter spasm, opisthotonus) to attacks of weakness and paralysis. Provided here is a short overview of the pathomechanisms behind such wide-ranging phenotypic presentations in these patients, followed by recommendations concerning the management of anesthesia in such populations.
authors.affiliation: Department of Anesthesia, Surgical Intensive Care, Prehospital Emergency Medicine and Pain Therapy, University Hospital Basel, Basel, Switzerland. Oliver.Bandschapp@usb.ch
authors.forename: Oliver
authors.initials: O
authors.lastname: Bandschapp
authors.forename: Paul A
authors.initials: PA
authors.lastname: Iaizzo
mesh_terms: D000758:Anesthesia; D053447:Channelopathies / Q000503:physiopathology; D006801:Humans; D007008:Hypokalemia / Q000503:physiopathology; D018482:Muscle, Skeletal / Q000503:physiopathology; D009224:Myotonia Congenita / Q000150:complications* / Q000503:physiopathology; D010245:Paralyses, Familial Periodic / Q000150:complications* / Q000503:physiopathology; D010347:Patient Care Planning; D012508:Sarcolemma / Q000502:physiology
keywords: anesthetic considerations; hyperkalemic and hypokalemic periodic paralysis; myotonia congenita